%0 Journal Article %A Hiroyuki Taniguchi %A Arata Azuma %A Takashi Ogura %A Yoshikazu Inoue %A Kingo Chida %A Masashi Bando %A Shinichi Kakutani %A Shuichiro Inagaki %A Moritaka Suga %A Yukihiko Sugiyama %A Shoji Kudoh %A Toshihiro Nukiwa %T Acute exacerbation of idiopathic pulmonary fibrosis: Analysis in a post-marketing surveillance of pirfenidone %D 2014 %J European Respiratory Journal %P 1902 %V 44 %N Suppl 58 %X Background: Acute exacerbation (AE) of idiopathic pulmonary fibrosis (IPF) is recognized as a common and critical event in the clinical course of IPF. However, the risk factors and association of AE with disease severity of IPF remains unclear.Aims and objectives: The aim of this study was to evaluate the risk factors and impact of disease severity on the frequency of AE.Methods: We analyzed 1342 patients with IPF who had been consecutively enrolled in a post-marketing surveillance of pirfenidone between Dec 2008 and Oct 2009 in Japan. In the population, the mean age was 69.5 ± 8.5 years with 76.4% males. Average baseline %VC was 66.1%, and 40% of patients were diagnosed as grade IV of disease severity according to Japanese Respiratory Society (JRS) criteria. AE-IPF was diagnosed by physicians according to the criteria in Japan (Taniguchi H. et al. Eur Respir J 2010; 35: 821-9). A Cox proportional hazards regression analysis was used to identify risk factors for AE-IPF.Results: Occurrence of AE-IPF was reported in total of 170 patients within 1 year of the observation, and 65% of AE-IPF cases died within 6 months after AE (median survival time after AE, 31 days). Multivariate analysis with Cox proportional hazards regression models showed that sex (male) [HR, 2.621; p=0.0031], %VC [HR, 0.976; p=0.0002] and high disease severity grade according to JRS criteria [HR, 1.341; p=0.0069] were risk factors for AE-IPF.Conclusions: This study suggests that sex (male), low %VC and high disease severity grade according to JRS criteria are risk factors for AE-IPF. Further analysis on the risk factors and the impact of disease severity will be presented. %U